Worldwide ancient lakes have been a major focal point of geological, biological, and ecological research, and key concepts in, for example, evolutionary biology are partly based on ancient lake studies. Ancient lakes can be found on most continents and climate zones with most actual or putative ancient lakes in Europe being restricted to the Balkan Region. The arguably most outstanding of them is the oligotrophic and karstic Lake Ohrid, a steep-sided graben of rift formation origin situated in the central Balkans. Here, an attempt is made to summarize current knowledge of the geological, limnological, and faunal history of Lake Ohrid. Additionally, existing data on biodiversity and endemism in Lake Ohrid are updated and evaluated, and patterns and processes of speciation are reviewed in the context of the Ohrid watershed, including its sister lake, Lake Prespa. Whereas the geological history of the Ohrid Graben is relatively well studied, there is little knowledge about the limnological and biotic history of the actual lake (e.g., the age of the extant lake or from where the lake first received its water, along with its first biota). Most workers agree on a time frame of origin for Lake Ohrid of 2-5 million years ago (Mya). However, until now, the exact limnological origin and the origin of faunal or floral elements of Lake Ohrid remain uncertain. Two largely contrasting opinions either favour the theory of de novo formation of Lake Ohrid in a dry polje with a spring or river hydrography or a palaeogeographical connection of Lake Ohrid to brackish waters on the Balkan Peninsula. Whereas neither theory can be rejected at this point, the data summarized in the current review support the de novo hypothesis. An assessment of the fauna and flora of Lake Ohrid confirms that the lake harbours an incredible endemic biodiversity. Despite the fact that some biotic groups are poorly studied or not studied at all, approximately 1,200 native species are known from the lake, including 586 animals, and at least 212 species are endemic, including 182 animals. The adjusted rate of endemicity is estimated at 36% for all taxa and 34% for Animalia. In terms of endemic biodiversity, Lake Ohrid is with these 212 known endemic species and a surface area of 358 km 2 probably the most diverse lake in the world, taking surface area into account. Preliminary phylogeographical analyses of endemic Lake Ohrid taxa indicate that the vast majority of respective sister taxa occurs in the Balkans and that therefore the most recent common ancestors of Ohrid-and non-Ohrid species likely resided in the region when Lake Ohrid came into existence. These data also indicate that there is relatively little faunal exchange and overlap between Lake Ohrid and its sister lake, Lake Prespa, despite the fact that the latter lake is a major water supplier for Lake Ohrid. Studies on selected species flocks and scatters, mostly in molluscs, point towards the assumption that only few lineages originally colonized Lake Ohrid from the Balkans and that the majority o...